297The Disabilities of the Arm, Shoulder and Hand (DASH) is a PRO-measuring
upper-extremity functional ability in musculoskeletal disease. Originally developed
by Hudak et al., it consists of a 30-item symptom and function scale [ 72 ]. All items
in the DASH are scored on a 5-point Likert scale, with 5 indicating extreme severity
or lack of function [ 73 ]. There is an abbreviated version, the QuickDASH [ 74 ]. In
SSc, the scale has been validated in Hungarian patients [ 75 ]. Both the DASH and
QuickDASH correlate with the HAQ-DI and SF-36, confi rming that much of the
disability in SSc is from the upper extremities [ 75 ].
Another PRO adapted for use in SSc is the 57-item ABILHAND questionnaire
[ 76 ]. The ABILHAND assesses the diffi culty of a variety of manual activities,
which the patient ranks from a scale of 0 to 5, with 0 indicating that the activity is
impossible and 5 that it is very easy. Patients do not rate tasks that they have never
performed [ 76 ]. Though it was originally developed in RA, it has been investigated
in SSc by asking patients to rank manual activities as impossible, diffi cult, or easy
[ 77 ]. The SSc-adapted ABILHAND was found to be reliable, valid, and reproduc-
ible, as well as linear and unidimensional [ 77 ].
The UK SSc Functional Score [ 53 ] and Michigan Hand Questionnaire [ 78 ] also
assess hand function; however, the latter may have limited use in SSc [ 28 ].
Additionally, several questions of the HAQ-DI and the SHAQ measure impairment
of the hands.
Mouth Disability
Many patients with SSc have oral problems, such as reduced oral opening, dry
mouth, altered appearance, diffi culty speaking, and impaired oral hygiene [ 28 ]. To
address these common concerns, Mouthon et al. created the Mouth Handicap Scale
in Systemic Sclerosis (MHISS) [ 79 ]. The MHISS has 12 self-reported items scored
from 0 to 4, which are summed. The MHISS has excellent test–retest reliability, and
good construct and divergent validity [ 79 ]. Three factors—reduced mouth opening,
dryness, and appearance concerns—have been found to explain nearly two-thirds of
the variance in scores [ 79 ]. MHISS scores also helped explain 36 % of HAQ score
variance, which could be due to the fact that severe SSc can cause both reduced
hand function and oral problems [ 79 ]. Thus, the assessment of oral disease in SSc is
important. The MHISS is the only tool that measures patient-reported mouth dis-
ability in SSc. Further research may include this questionnaire, especially if oral
health is being studied in SSc.
Pain
Pain in SSc is likely underrecognized, despite its occurrence in more than 80 % of
patients. On average, patients reported their pain at 4 out of 10 [ 15 ]. Therefore, pain is
an important outcome to measure. Pain in SSc is usually assessed by using VAS, Likert
scales, or other change scales. The scales may assess global pain or problem- specifi c
11 PROMs for Systemic Sclerosis (Scleroderma)